Early cognitive decline in Creutzfeldt-Jakob disease associated with human growth hormone treatment. Issue 10 (20th October 2003)

• Record Type: Journal Article
• Authors: Cordery, R J; Hall, M; Cipolotti, L; Al-Sarraj, S; O'Donovan, D G; Davidson, L; Adlard, P; Rossor, M N
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 74:Issue 10(2003)
• Page Start: 1412
• Page End: 1416
• Publication Date: 2003-10-20
• Subjects: Creutzfeldt-Jakob disease -- human growth hormone -- neuropsychology; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.74.10.1412
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Background: Most cases of Creutzfeldt-Jakob disease (CJD) in recipients of human cadaveric growth hormone present with a cerebellar syndrome. Dementia is thought to occur late and as a minor feature of the illness. However, neuropsychology data published on these cases are largely qualitative and anecdotal. The first published case does include a neuropsychological assessment seven months after the onset of a cerebellar syndrome, showing evidence of intellectual decline. Subsequent reports hint that cognitive problems may be present in the initial stages of the illness. Objective: To assess early cognition in Creutzfeldt-Jakob disease in recipients of pituitary derived human growth hormone. Methods: Detailed neuropsychology assessment is reported at referral (mean 4.5 months from the onset of symptoms; range 4 to 6 months) in five patients with histologically proven human growth hormone derived CJD. Results: All cases presented with a cerebellar syndrome and only one had noticed mild memory problems. On formal testing, however, four had demonstrable mild intellectual decline, as measured on the WAIS-R. One case showed selective visual memory impairment and frontal executive dysfunction. Conclusions: These findings suggest that, although not the presenting feature, mild cognitive decline may be evident in the early stages of CJD associated with human cadaveric growth hormone treatment.